Immunodeficiency Flashcards
what is primary immunodeficiency?
congenital-inherited as autosomal recessive genes
what is secondary immune deficiency?
compromised by outside factors (cancer, immuno-suppression, HIV, malnutrition, stress, trauma, excessive exercise)
what are some signs of a immunodeficiency disorder?
persistence, recurrent infections, poor response to treatment, delayed recovery, certain cancers and infections from things that do not usually cause infection
recurrent bacterial infections is a sign of deficiency of:
B Cells
intracellular, viral, bacterial, fungal, or protozoal infections could be due to a deficiency of:
T cells
systemic infection with bacteria that are normally non infectious could be caused by a def in:
phagocytes
bacterial infections and autoimmune disease can be caused by def in:
complement proteins
a lack in leukocyte adhesion molecules could lead to
recurrent bacterial infections, impaired pus formation and impaired wound healing
chart on 363
common immunodef
what ca immunoelectrophoretic be used for?
absence or abnormal levels of antibody/immunoglobulin or complement proteins
what is X linked agammaglobulima?
o Mutation in Bruton’s tyrosine kinase (Btk) gene that leads to a severe blockade of (at the pre-B cell development to immature B cell (85%, boys)
o No circulating B cells - All classes of Ig are absent
o Lack of cells with B cell marker CD19 and/or CD20
what is Transient hypogammaglobulinemia?
o More likely to occur in premature babies
o Temporary - 5% of infants are symptomatic when younger than 6 months, 50% become
symptomatic at 6-12 months, and 25% become symptomatic when they are older than 12
months, increasing to the normal reference range by age 2-6 years
what is Common variable hypogammaglobulinemia
characterized by?
o Low levels - decreased
o B cells fail to mature into plasma cells – has normal numbers of the cells that produce
antibodies (B-lymphocytes), but these cells fail to undergo normal maturation into plasma cells
o Variable – Some patients have a decrease in both IgG and IgA, whereas others have a decrease
in all three major types of immunoglobulins (IgG, IgA, and IgM)
what is the most common Selective immunoglobulin deficiency?
IgA
what immuno effect happens due to IgA deficiency?
–Decreased immune function in the mucosal surfaces in the mouth, gastrointestinal tract (stomach and intestines), and lungs.
–This deficiency results in an increased risk for respiratory and gastrointestinal infections.
what are clinical symptoms of IgA deficiency?
-family history of IgA deficiency
-mouth infections
-frequent respiratory infections
-unexplained asthma or bronchitis
-chronic diarrhea
what is the treatment for IgA deficency?
- Antibiotics and anti-inflammatories.
- Intravenous immunoglobulin (IVIG) not recommended - increased risk of life-threatening allergic reactions, or anaphylaxis, when they receive blood products containing some IgA.
what does lack of a precipitant line on a serum immunoelectrophoretic test?
deficncy of what is being tested
what is congenital thymic aplasia (DiGeorge)
- Thymus abnormal – Poor T cell development
- Parathyroid abnormal - blood calcium is low in routine blood tests
- Infant may be “jittery” or have seizures (convulsions) due to the low
calcium. - High incidence of autoimmunity
Chronic mucocutaneous candidiasis is an example of what type of disease?
T lymphocyte disorder
what is Ataxia telangiectasia?
Autosomal recessive chromosomal breakage disorder of the ATM (ataxia telangiectasia mutated) protein, an essential enzyme for DNA repair
o Progressive decline of motor neurologic functions
what is Severe combined deficiencies disease
(SCID)?
bubble boy syndrome
* Severe deficiencies in both T-cell and B-cell functions.
* Defects lead to extreme susceptibility to severe infections
what are the two main causes of SCID?
- Adenosine Deaminase (50%)
- Recombinase activating genes 1 and 2 (RAG1 and RAG2) deficiency
what are signs of SCIDs in infants
severe infections
susceptible to vaccines
fungal infections
persistent diarrhea
severe weight loss
rashes (mothers T cells against baby tissues)
what is the most successful treatment for SCIDs?
bone marrow transplantation
what is chediak higashi syndrome
- Adenosine Deaminase (50%)
- Recombinase activating genes 1 and 2 (RAG1 and RAG2) deficiency
what are the symptoms of chediak higashi syndrome?
partial albinism
platelet dysfunction
aggressive periodontitis
what is chronic granulomatous disease?
The body’s phagocytic cells are unable to make hydrogen peroxide and other oxidants needed to kill certain microorganisms.
-Patients susceptible to infections with bacteria and fungi that usually are of low virulence
what are the symptoms of chronic granulomatous disease?
in infancy recurrent unusual infection
prolonged drainage
what are some complications of deficiency in complement cascades?
Increased susceptibility to bacterial infections and
autoimmune diseases
Failure to clear immune complex and causes
symptoms that resemble lupus (SLE)
Deficiencies of C5, C6, C7, C8, or C9, late-acting
complement proteins involved in forming holes in
membranes.
–edema
